François Grand'Maison

Abstract: All disorders of neuromuscular transmission (NMT) may cause ventilatory failure, albeit rarely. Respiratory muscle weakness is occasionally the presenting feature of myasthenia gravis (MG), the Lambert-Eaton myasthenic syndrome (LEMS), hypermagnesemia and botulism. Chronic MG, congenital myasthenic syndromes and LEMS may be acutely exacerbated by various intercurrent conditions and by drugs which interfere with NMT. Finally, in the ICU, difficulty in weaning from the ventilator may be caused by prolonged use of neuromuscular blocking agents. Electrophysiological studies of NMT disorders in the intensive care unit have rarely been reported. Nevertheless, the available data indicates that the electrodiagnosis of severe NMT disorders can be misleading. With severe NMT defects, the electrophysiological distinction between post-synaptic and pre-synaptic disorders is blurred and the differential diagnosis with myopathies may be difficult. A clinically suspected NMT disorder should therefore not be ruled out when electrodiagnosis fails to demonstrate the expected abnormalities.

Can. J. Neurol. Sci. 1998; 25: S36 - S39